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https://www.readbyqxmd.com/read/27913462/pure-red-cell-aplasia
#1
Robert T Means
Pure red cell aplasia (PRCA) is a syndrome defined by a normocytic normochromic anemia with severe reticulocytopenia and marked reduction or absence of erythroid precursors from the bone marrow. Diamond-Blackfan anemia is a congenital form of PRCA. Acquired PRCA may be either a primary disorder or secondary to some other disorder or agent. Primary acquired PRCA is an autoimmune disorder that is frequently antibody-mediated. Myelodysplastic syndromes may also present with the morphologic appearance of PRCA. Secondary acquired PRCA may be associated with collagen vascular/autoimmune disorders such as systemic lupus erythematosus; lymphoproliferative disorders such as chronic lymphocytic leukemia or large granular lymphocyte leukemia; infections, particularly B19 parvovirus; thymoma and other solid tumors; or a variety of other disorders, drugs, or toxic agents...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27902603/purulent-lupus-panniculitis-unmasked-by-fdg-pet-ct-scan-a-case-report
#2
Kornelis S M van der Geest, Rada V Moerman, Klaas P Koopmans, Nicole D Holman, Wilbert M T Janssen
RATIONALE: Lupus panniculitis (LP) is a unique variant of cutaneous lupus erythematosus. Clinical manifestations are typically mild and include erythema, nodules, and small ulcers. In certain cases, diagnosing LP may be challenging. Skin overlying the typical subcutaneous inflammation may appear normal, and bacterial superinfections of the skin sometimes mask the underlying LP. It has been suggested that a computed tomography (CT) scan may help to identify obscure LP lesions. Here, we report a case of a 54-year-old woman with an unusually severe form of LP, in which the full disease extent was only revealed by a fluorodeoxyglucose positron emission tomography (FDG-PET)/CT scan...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27899525/reactive-oxygen-species-induce-virus-independent-mavs-oligomerization-in-systemic-lupus-erythematosus
#3
Iwona A Buskiewicz, Theresa Montgomery, Elizabeth C Yasewicz, Sally A Huber, Michael P Murphy, Richard C Hartley, Ryan Kelly, Mary K Crow, Andras Perl, Ralph C Budd, Andreas Koenig
The increased expression of genes induced by type I interferon (IFN) is characteristic of viral infections and systemic lupus erythematosus (SLE). We showed that mitochondrial antiviral signaling (MAVS) protein, which normally forms a complex with retinoic acid gene I (RIG-I)-like helicases during viral infection, was activated by oxidative stress independently of RIG-I helicases. We found that chemically generated oxidative stress stimulated the formation of MAVS oligomers, which led to mitochondrial hyperpolarization and decreased adenosine triphosphate production and spare respiratory capacity, responses that were not observed in similarly treated cells lacking MAVS...
November 29, 2016: Science Signaling
https://www.readbyqxmd.com/read/27888486/a-slowly-growing-orange-patch-on-the-cheek-diagnosis-of-lupus-vulgaris-20-years-after-onset-of-first-skin-changes
#4
Claudia Pföhler, Michael Klotz, Thomas Wehler, Thomas Vogt, Cornelia S L Müller
Tuberculosis is a worldwide occurring disease that affects approximately 20-40% of the world's population and in particular in developing countries. However, in times of migration, industrialised countries are again being more and more affected. Cutaneous tuberculosis is rare and lupus vulgaris represents its most common form. Preferentially, young adults in developing or low-income countries are affected from cutaneous tuberculosis, which usually occurs in previously sensitized persons with a high degree of tuberculin sensitivity and a good immunoresponse...
November 25, 2016: Dermatology and Therapy
https://www.readbyqxmd.com/read/27884116/kidney-disease-in-the-elderly-biopsy-based-data-from-14-renal-centers-in-poland
#5
Agnieszka Perkowska-Ptasinska, Dominika Deborska-Materkowska, Artur Bartczak, Tomasz Stompor, Tomasz Liberek, Barbara Bullo-Piontecka, Anna Wasinska, Agnieszka Serwacka, Marian Klinger, Jolanta Chyl, Michal Kuriga, Robert Malecki, Krzysztof Marczewski, Bogdan Hryniewicz, Tadeusz Gregorczyk, Monika Wieliczko, Stanislaw Niemczyk, Olga Rostkowska, Leszek Paczek, Magdalena Durlik
BACKGROUND: Longer life expectancy is associated with an increasing prevalence of kidney disease. Aging itself may cause renal damage, but the spectrum of kidney disorders that affect elderly patients is diverse. Few studies, mostly form US, Asia and West Europe found differences in the prevalence of some types of kidney diseases between elderly and younger patients based on renal biopsy findings, with varied proportion between glomerulopathies and arterionephrosclerosis as a dominant injury found...
November 25, 2016: BMC Nephrology
https://www.readbyqxmd.com/read/27881371/pure-red-cell-aplasia
#6
REVIEW
Robert T Means
Pure red cell aplasia (PRCA) is a syndrome defined by a normocytic normochromic anemia with severe reticulocytopenia and marked reduction or absence of erythroid precursors from the bone marrow. Diamond-Blackfan anemia is a congenital form of PRCA. Acquired PRCA may be either a primary disorder or secondary to some other disorder or agent. Primary acquired PRCA is an autoimmune disorder that is frequently antibody-mediated. Myelodysplastic syndromes may also present with the morphologic appearance of PRCA. Secondary acquired PRCA may be associated with collagen vascular/autoimmune disorders such as systemic lupus erythematosus; lymphoproliferative disorders such as chronic lymphocytic leukemia or large granular lymphocyte leukemia; infections, particularly B19 parvovirus; thymoma and other solid tumors; or a variety of other disorders, drugs, or toxic agents...
November 24, 2016: Blood
https://www.readbyqxmd.com/read/27870763/the-effect-of-psychosocial-and-neuropsychiatric-factors-on-medication-adherence-in-a-cohort-of-women-with-systemic-lupus-erythematosus
#7
Cindy Flower, Ian Hambleton, Mike Campbell
BACKGROUND: Medication adherence in systemic lupus erythematosus (SLE) reduces disease activity and the risk of flares. OBJECTIVES: We evaluated adherence in women with SLE who exhibit high morbidity and mortality. We evaluated demographic data and 2 conventional adherence predictors: self-efficacy and health literacy, along with 2 potential neuropsychiatric SLE complications: cognitive dysfunction and depression. METHODS: One hundred six women randomly selected from the Barbados National Lupus Registry completed the Self-efficacy for Appropriate Medication Use Scale, Rapid Estimate of Adult Literacy in Medicine-Short Form, Cognitive Symptom Inventory, Beck Depression Inventory II, and Morisky's Medication Adherence Questionnaire (MAQ)...
December 2016: Journal of Clinical Rheumatology: Practical Reports on Rheumatic & Musculoskeletal Diseases
https://www.readbyqxmd.com/read/27867381/a-critical-reappraisal-of-neutrophil-extracellular-traps-and-netosis-mimics-based-on-differential-requirements-for-protein-citrullination
#8
Maximilian F Konig, Felipe Andrade
NETosis, an antimicrobial form of neutrophil cell death, is considered a primary source of citrullinated autoantigens in rheumatoid arthritis (RA) and immunogenic DNA in systemic lupus erythematosus (SLE). Activation of the citrullinating enzyme peptidylarginine deiminase type 4 (PAD4) is believed to be essential for neutrophil extracellular trap (NET) formation and NETosis. PAD4 is therefore viewed as a promising therapeutic target to inhibit the formation of NETs in both diseases. In this review, we examine the evidence for PAD4 activation during NETosis and provide experimental data to suggest that protein citrullination is not a universal feature of NETs...
2016: Frontiers in Immunology
https://www.readbyqxmd.com/read/27855151/advances-in-the-care-of-children-with-lupus-nephritis
#9
Scott E Wenderfer, Natasha M Ruth, Hermine I Brunner
The care of children with lupus nephritis (LN) has changed dramatically over the past 50 years. The majority of patients with childhood-onset systemic lupus erythematosus (cSLE) develop LN. In the 1960's, prognosis in children was worse than in adults; therapies were limited and toxic. Nearly half of cases resulted in death within 2-years. Since this time, several diagnostic recommendations and disease-specific indices have been developed to assist physicians caring for patients with LN. Pediatric researchers are validating and adapting these indices and guidelines for the treatment of LN in cSLE...
November 17, 2016: Pediatric Research
https://www.readbyqxmd.com/read/27846944/primary-cicatricial-alopecia-lymphocytic-primary-cicatricial-alopecias-including-chronic-cutaneous-lupus-erythematosus-lichen-planopilaris-frontal-fibrosing-alopecia-and-graham-little-syndrome
#10
REVIEW
Chantal Bolduc, Leonard C Sperling, Jerry Shapiro
Both primary and secondary forms of cicatricial alopecia have been described. The hair follicles are the specific target of inflammation in primary cicatricial alopecias. Hair follicles are destroyed randomly with surrounding structures in secondary cicatricial alopecia. This 2-part continuing medical education article will review primary cicatricial alopecias according to the working classification suggested by the North American Hair Research Society. In this classification, the different entities are classified into 3 different groups according to their prominent inflammatory infiltrate (ie, lymphocytic, neutrophilic, and mixed)...
December 2016: Journal of the American Academy of Dermatology
https://www.readbyqxmd.com/read/27843374/potential-serum-and-urine-biomarkers-in-patients-with-lupus-nephritis-and-the-unsolved-problems
#11
REVIEW
Song-Chou Hsieh, Chang-Youh Tsai, Chia-Li Yu
Lupus nephritis (LN) is one of the most frequent and serious complications in the patients with systemic lupus erythematosus. Autoimmune-mediated inflammation in both renal glomerular and tubulointerstitial tissues is the major pathological finding of LN. In clinical practice, the elevated anti-dsDNA antibody titer concomitant with reduced complement C3 and C4 levels has become the predictive and disease-activity surrogate biomarkers in LN. However, more and more evidences suggest that autoantibodies other than anti-dsDNA antibodies, such as anti-nucleosome, anti-C1q, anti-C3b, anti-cardiolipin, anti-endothelial cell, anti-ribonuclear proteins, and anti-glomerular matrix (anti-actinin) antibodies, may also involve in LN...
2016: Open Access Rheumatology: Research and Reviews
https://www.readbyqxmd.com/read/27831941/linear-sclerodermoid-lupus-erythematosus-profundus-in-a-child
#12
Amira Elbendary, John Griffin, Suzanne Li, Brook Tlougan, Jacqueline M Junkins-Hopkins
Lupus erythematosus panniculitis, also known as lupus profundus, is a variant in the clinicopathological spectrum of lupus erythematosus (LE) affecting about 2%-3% of LE patients. A linear configuration of LE panniculitis has been reported rarely with rare reports describing the coexistence of different forms of cutaneous LE and localized morphea. In this study, the authors present a 9-year-old girl with linear arrangement of subcutaneous nodules on her left forearm. Microscopic findings from 2 biopsies included lymphocytes at the dermoepidermal junction with mild interface dermatitis, a dense lymphocytic infiltrate that was concentrated around adnexae and subcutaneous fat in concert with thickened collagen bundles and mild widening of fibrous septae surrounding fat lobules...
December 2016: American Journal of Dermatopathology
https://www.readbyqxmd.com/read/27830539/primary-autoimmune-myelofibrosis-a-case-report-and-review-of-the-literature
#13
Yasmin Abaza, C Cameron Yin, Carlos E Bueso-Ramos, Sa A Wang, Srdan Verstovsek
Autoimmune myelofibrosis is a rare, distinct clinicopathological entity that can occur in isolation (primary) or in association with systemic autoimmune disorders (secondary), such as systemic lupus erythematosus and Sjogren's syndrome. This disease is characterized by isolated or combined chronic cytopenias associated with autoimmune phenomena and bone-marrow fibrosis. Due to the rarity of this disease, patients are frequently misdiagnosed as having primary myelofibrosis, the most common form of bone-marrow fibrosis...
November 9, 2016: International Journal of Hematology
https://www.readbyqxmd.com/read/27829463/frequency-and-predictors-of-the-lupus-low-disease-activity-state-in-a-multi-national-and-multi-ethnic-cohort
#14
Vera Golder, Rangi Kandane-Rathnayake, Alberta Yik-Bun Hoi, Molla Huq, Worawit Louthrenoo, Yuan An, Zhan Guo Li, Shue Fen Luo, Sargunan Sockalingam, Chak Sing Lau, Alfred Lok Lee, Mo Yin Mok, Aisha Lateef, Kate Franklyn, Susan Morton, Sandra Teresa V Navarra, Leonid Zamora, Yeong-Jian Wu, Laniyati Hamijoyo, Madelynn Chan, Sean O'Neill, Fiona Goldblatt, Eric Francis Morand, Mandana Nikpour
BACKGROUND: Systemic lupus erythematosus (SLE) is a chronic heterogeneous disease with considerable burden from disease activity and damage. A novel clinical treatment target in the form of the lupus low disease activity state (LLDAS) has been recently reported, with retrospective validation showing that time spent in LLDAS translates to reduced damage accrual. The objectives of this study were to describe the frequency and identify the predictors of attaining LLDAS in a large multinational cohort of patients with SLE...
November 9, 2016: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/27821515/an-autosomal-recessive-dnase1l3-related-autoimmune-disease-with-unusual-clinical-presentation-mimicking-systemic-lupus-erythematosus
#15
A Carbonella, G Mancano, E Gremese, F S Alkuraya, N Patel, F Gurrieri, G Ferraccioli
We describe the third family in the world, after Arabian and Turkish ones, displaying an autosomal recessive autoimmune disease (AID), mimicking systemic lupus erythematosus (SLE), with unusual manifestations due to a homozygous frame-shift variant in DNASE1L3 SLE is a complex AID characterized by multiple organ involvement. Genetic risk variants identified account for only 15% of SLE heritability. Rare Mendelian forms have been reported, including DNASE1L3-related SLE.Through specific genetic tests we identified a homozygous 2 bp-deletion c...
November 7, 2016: Lupus
https://www.readbyqxmd.com/read/27816459/microrna-21-microrna-181a-and-microrna-196a-as-potential-biomarkers-in-adult-egyptian-patients-with-systemic-lupus-erythematosus
#16
Tarek K Motawi, Dahlia A Mohsen, Shohda A El-Maraghy, Mona A Kortam
Dysregulation of miRNAs has been described in systemic lupus erythematosis (SLE), however the clinical relevance of using miRNAs as biomarkers for SLE or predictors of disease progression is poorly investigated. This study investigated the expression signature of plasma miR-21, miR-181a and miR-196a among seventy SLE patients with different systemic lupus erythematosis disease activity index (SLEDAI) scores and thirty healthy controls using quantitative real-time PCR. Plasma IL-10 level was also measured in patients and control groups...
November 2, 2016: Chemico-biological Interactions
https://www.readbyqxmd.com/read/27788504/repeated-renal-biopsy-a-predictive-tool-to-assess-the-probability-of-renal-flare-in-lupus-nephritis
#17
Gastón J Piñeiro, Pilar Arrizabalaga, Manel Solé, Rosa M Abellana, Gerard Espinosa, Ricard Cervera
BACKGROUND: How one responds to treatment of lupus nephritis (LN) is based on clinical features, but the activity in renal biopsy (RB) is uncertain. We have described the therapeutic decisions after performing a repeated RB on the assessment of response to intravenous cyclophosphamide (IC) and the possible prognostic role of this repeated RB. METHODS: Clinical, laboratory and histological features at the initial RB and repeated RB were analyzed in 35 patients. RESULTS: Data in the initial versus the repeated RB were serum creatinine 1...
October 28, 2016: American Journal of Nephrology
https://www.readbyqxmd.com/read/27782255/systemic-lupus-erythematosus-molecular-cloning-and-analysis-of-recombinant-monoclonal-kappa-light-chain-ngta1-me-pro-with-two-metalloprotease-active-centers
#18
Anna M Timofeeva, Valentina N Buneva, Georgy A Nevinsky
It was shown previously that approximately 30% ± 5% of antibodies against myelin basic protein (MBP) and the DNA of patients with systemic lupus erythematosus (SLE) and multiple sclerosis (MS) possess catalytic activities that play an important negative role in the pathogenesis of MS and SLE. An immunoglobulin light chain phagemid library derived from peripheral blood lymphocytes of patients with SLE was used. The small pools of phage particles displaying light chains with different affinity for MBP were isolated by affinity chromatography on MBP-Sepharose, and the fraction eluted with 0...
October 26, 2016: Molecular BioSystems
https://www.readbyqxmd.com/read/27760048/mtor-has-distinct-functions-in-generating-versus-sustaining-humoral-immunity
#19
Derek D Jones, Brian T Gaudette, Joel R Wilmore, Irene Chernova, Alexandra Bortnick, Brendan M Weiss, David Allman
Little is known about the role of mTOR signaling in plasma cell differentiation and function. Furthermore, for reasons not understood, mTOR inhibition reverses antibody-associated disease in a murine model of systemic lupus erythematosus. Here, we have demonstrated that induced B lineage-specific deletion of the gene encoding RAPTOR, an essential signaling adaptor for rapamycin-sensitive mTOR complex 1 (mTORC1), abrogated the generation of antibody-secreting plasma cells in mice. Acute treatment with rapamycin recapitulated the effects of RAPTOR deficiency, and both strategies led to the ablation of newly formed plasma cells in the spleen and bone marrow while also obliterating preexisting germinal centers...
November 1, 2016: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/27752336/reduction-in-erythrocyte-bound-complement-activation-products-and-titres-of-anti-c1q-antibodies-associate-with-clinical-improvement-in-systemic-lupus-erythematosus
#20
Jill Buyon, Richard Furie, Chaim Putterman, Rosalind Ramsey-Goldman, Kenneth Kalunian, Derren Barken, John Conklin, Thierry Dervieux
BACKGROUND: The relationship between cell-bound complement activation products (CB-CAPs: EC4d, EC3d), anti-C1q, soluble complement C3/C4 and disease activity in systemic lupus erythematosus (SLE) was evaluated. METHODS: Per protocol, at baseline all SLE subjects enrolled in this longitudinal study presented with active disease and elevated CB-CAPs. At each monthly visit, the non-serological (ns) Safety of Estrogens in Lupus Erythematosus: National Assessment (SELENA-SLEDAI) and the British Isles Lupus Assessment Group (BILAG)-2004 index scores were determined as was a random urinary protein to creatinine ratio (uPCR)...
2016: Lupus Science & Medicine
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